Browsing by Author "Lichtner, A"
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- Cytomegalovirus (CMV) and transfusion therapy: prevalence of cmv seropositivity in a portuguese blood donor population (2007-2014)Publication . Cardoso, E; Lichtner, A; Barra, A; Costa, C; Plácido, C; Nunes, C; Ferreira, M; Rebelo, S; Santos, C; Mota, MBackground: Cytomegalovirus (CMV or human herpesvisrus-5) is a common infection and not clinically significant in immunocompetent individuals. It’s the most important herpesvirus with reference to transfusion and, as all human herpesvirus, has the capability to lie dormant in tissues after an acute infection. The risk of CMV infectivity is still a major problem in immunocompromised patients requiring transfusion therapy, and should be minimized in CMV-negative pregnant women, fetuses, premature infants and neonats, transplant recipients and other severely immunosuppressed patients. The residual risk of transfusion-transmitted CMV infection is between 2.3% and 3% for leucocyte-reduced blood components, and the additional use of anti-CMV screened blood components decreases this risk to less than 1%, which, in our point of view, justifies the non-abandonment of CMV-seronegative blood bank inventories, especially in high prevalent populations. There is substantial variation in the donor rates of CMV seropositivity described in the literature (20-95%) and it seems to be inversely related to improved hygiene and living conditions. The same principle applies to the seroconversion rate per year. Aims: To determine the prevalence of CMV seropositivity and CMV seroconversion rate per year in a Portuguese blood donor population over an eight-year period of time (2007-2014), and compare it to those mentioned in other studies. Methods: Blood samples from donors were analyzed during the period 2007-2014 (8 years), and tested for detection of anti-CMV antibodies (‘total’ anti-CMV ELISA-based assays, capable of detecting both IgG and IgM class antibodies- Siemens Enzygnost® Anti-CMV/IgG+IgM), as they belonged to a first-time or to a previously tested CMV-seronegative donor. The prevalence of CMV seropositivity and the seroconversion rate per year were retrospectively determined among this blood donors population. Results: A total of 42.286 blood collections were analyzed. The prevalence of CMV infection was determined for each year: 2007 – 86.4%; 2008 – 87,2%; 2009 – 86.5%; 2010 – 88.9%; 2011 – 90.2%; 2012 – 89.7%; 2013 – 91,3%; 2014 – 89,4%. The CMV seroconversion rate per year was 1.43% (average age of seroconversion 36.4 years old; 81.8% men and 18,2% women). Summary/Conclusions: All blood components transfused in Portugal are leucocyte-reduced. We try to provide CMV seronegative blood components to all patients in high risk CMV infection, even in a high prevalent population as ours. The existence of CMV-seronegative blood bank inventories does not reflect practice countrywide, but is still important for some high-risk groups of patients, and allows us to respond to the needs of our hospital and other institutions (whenever possible).
- A flebotomia, co-adjuvante terapêutico em doente com HCC, PCT e sobrecarga de ferroPublication . Plácido, C; Barra, A; Lichtner, A; Cardoso, E; Costa, C; Nunes, CIntrodução A Porfíria Cutânea Tarda (PCT) é a forma mais frequente de Porfíria a nível mundial, podendo ser hereditária, com uma prevalência variável, entre 1:5000 – 1:70.000 (1) ou adquirida, sendo a Hepatite C Crónica (HCC) um dos factores predisponentes. Os doentes infectados têm uma maior predisposição para o desenvolvimento de alterações do metabolismo da porfirina, com alterações cutâneas que podem limitar a sua actividade, e sobrecarga de ferro. (1) A prevalência mundial da infecção pelo Vírus da Hepatite C (VHC) em doentes com PCT é de 47%, sendo o genótipo 1b o mais prevalente (~90%).(2) A flebotomia é um tratamento que permite tanto a redução de ferro como das porfirinas dos tecidos, diminuindo os seus efeitos deletérios.(1) A literatura refere que o tratamento, com interferão, dos doentes com HCC, pode levar a melhoria das manifestações cutâneas, bem como à normalização das porfirinas na urina, enzimas hepáticas e valores da ferritina.(2) Objectivo Partilhar um caso clínico de PCT em doente com HCC, e o papel da flebotomia como terapêutica coadjuvante. Caso Clínico Homem, 52 anos, caucasiano, ex-consumidor de drogas EV e HCC. Seguido em Dermatologia, por PCT (medicado com hidroxicloroquina – 400mg/semana), e Hepatologia, candidato a terapêutica tripla com Pegintron®, Ribavarina e Bocepravir. Análises iniciais: Porfirinas na urina 2015 µg/24h (<150); ferritina 458,2ng/ml (22-322); Sat. Transferrina 44 % (26-42); AST 68 U/L (0-34); ALT 125 U/L (10-49); GGT 151 U/L (<73); Htc 51,3%; Carga viral VHC 893563 UI/mL (Log 10 – 5,95). Genótipo 1b. Polimorfismo para a IL28B TT. Fibroscan®: Cirrose hepática. Referenciado à consulta de imuno-hemoterapia, para tratamento complementar com flebotomias, onde foi seguido e tratado durante 9 meses (9 flebotomias), com remissão total das lesões cutâneas, secundárias a PCT e valores finais de ferritina de 13 ng/ml. Fez terapêutica tripla durante 1 ano (posteriormente ARN viral não detectável). Última consulta imunohemoterapia: Ferritina 54ng/ml, coproporfirinas totais na urina 29 µg/24h (<100 µg/24h), AST 20U/L, ALT 15U/L e GGT 33U/L. Discussão/Conclusão Neste caso, a flebotomia revelou ser uma terapêutica eficaz na redução da sintomatologia cutânea da PCT e de diminuição da sobrecarga de ferro, não tendo limitado o início de terapêutica tripla para o VHC.
- Importância da genotipagem na terapêutica transfusional de doentes com drepanocitosePublication . Costa, C; Lichtner, A; Rodrigues, MJ; Moser, MI; Barra, A; Cardoso, E; Fernandes, A; Magalhães, D; Nunes, C; Pereira, M; Plácido, C; Simões, AIntrodução A drepanocitose é uma hemoglobinopatia autossómica recessiva com uma incidência na população portuguesa de 0,32%. A História, associada à actual imigração de países africanos de língua portuguesa, deram origem a alguns “hot spots” de portadores no centro e sul do país e à considerável incidência da drepanocitose. A fenotipagem alargada tem sido utilizada para transfundir com segurança os doentes com drepanocitose, prevenindo a aloimunização e as reacções transfusionais hemolíticas. Nos últimos anos, contudo, vários estudos demonstraram a importância da genotipagem na transfusão destes doentes. O fenótipo Fy(a-b-), comum em Africanos e raro noutras populações, na maioria dos casos tem como origem genética, uma mutação no promotor do gene FYB (FY*null01), que impede a transcrição do antigénio Fyb nos glóbulos vermelhos mas mantêm intacta a sua expressão noutros tecidos. Assim, sempre que se detecta essa mutação, o doente pode ser transfundido com sangue Fyb+ sem risco de imunização Objectivo Estudo molecular dos drepanocíticos relativamente aos principais antigénios dos Sistemas Kell, Kidd e Duffy para transfundir com maior segurança evitando a aloimunização. Métodos Foram genotipados 21 drepanocíticos politransfundidos durante o ano de 2014, com o Kit KKD-Type BAGHealthcare, Alemanha. Os doentes eram todos Afro-Portugueses, 13 mulheres e 8 homens, com idades entre os 2 e os 30 anos de idade. Resultados Os resultados serológicos dos fenótipos Kell, Kidd e Duffy foram confirmados pelos estudos moleculares. No Sistema Duffy foram identificados 3 fenótipos e a genotipagem revelou a presença de FY*null01 em 19 doentes (90%), sendo a maioria destes Fy(a-b-) (17 - 89,5%) e os restantes Fy(a+b-) (2-10,5%). Conclusão A genotipagem demonstrou ser importante para a selecção mais adequada de Concentrados Eritrocitários (CEs), evitando a aloimunização em doentes drepanocíticos. Os resultados demonstraram que 90% dos doentes drepanocíticos estudados possuíam a mutação no promotor do gene FYB, tornando possível a transfusão de CEs Fyb+ a doentes com os fenótipos Fy(a-b-) e Fy(a+b-). Esta característica permitiu-nos aumentar a base de dadores compatíveis com estes doentes pois a incidência de dadores Fy(a-b-) é muito baixa na nossa população.
- Partial Red Blood Cell Exchange in Children and Young Patients with Sickle Cell Disease: Manual Versus Automated Procedure.Publication . Escobar, C; Moniz, M; Nunes, P; Abadesso, C; Ferreira, T; Barra, A; Lichtner, A; Loureiro, H; Dias, A; Almeida, HINTRODUCTION: The benefits of manual versus automated red blood cell exchange have rarely been documented and studies in young sickle cell disease patients are scarce. We aim to describe and compare our experience in these two procedures. MATERIAL AND METHODS: Young patients (≤ 21 years old) who underwent manual- or automated-red blood cell exchange for prevention or treatment of sickle cell disease complications were included. Clinical, technical and hematological data were prospectively recorded and analyzed. RESULTS: Ninety-four red blood cell exchange sessions were performed over a period of 68 months, including 57 manual and 37 automated, 63 for chronic complications prevention, 30 for acute complications and one in the pre-operative setting. Mean decrease in sickle hemoglobin levels was higher in automated-red blood cell exchange (p < 0.001) and permitted a higher sickle hemoglobin level decrease per volume removed (p < 0.001), while hemoglobin and hematocrit remained stable. Ferritin levels on chronic patients decreased 54%. Most frequent concern was catheter outflow obstruction on manual-red blood cell exchange and access alarm on automated-red blood cell exchange. No major complication or alloimunization was recorded. DISCUSSION: Automated-red blood cell exchange decreased sickle hemoglobin levels more efficiently than manual procedure in the setting of acute and chronic complications of sickle cell disease, with minor technical concerns mainly due to vascular access. The threshold of sickle hemoglobin should be individualized for clinical and hematological goals. In our cohort of young patients, the need for an acceptable venous access was a limiting factor, but iron-overload was avoided. CONCLUSION: Automated red blood cell exchange is safe and well tolerated. It permits a higher sickle hemoglobin removal efficacy, better volume status control and iron-overload avoidance.
- Reverting vitamin k antagonists with prothrombin complex concentrate: a three-year retrospective studyPublication . Plácido, C; Barra, A; Lichtner, A; Cardoso, E; Costa, C; Nunes, CBackground Correcting coagulopathy has been a difficult challenge for hematologists throughout the world. Practice guidelines recommend vitamin K for the reversal of anticoagulation in asymptomatic patients with elevated INR, in patients who require surgery and in patients with serious bleeding. Prothrombin Complex Concentrate (PCC) is having progressive importance in bleeding management and reversal of International Normalized Ratio´s (INR) in patients taking oral vitamin K antagonists (VKA). Aims The main objective of our study is to determine the efficiency of PCC in the correction of INR in patients taking oral VKA and try to understand if the administration of vitamin K plays a role in the survival rates in these patients. Methods We carried out a retrospective, unicenter, descriptive study that included 54 patients taking oral VKA that required Octaplex ® administration in order to try to control bleeding and/or correct INR. Of these patients 55% were female and 45% male. Average age was 76 years old. The data was collected from March 2011 to November 2014. The INR results were collected before and after the treatment with PCC. Results 87% of the studied patients (47 patients) were taking warfarin and 13% (7 patients) acenocoumarol. The main cause of treatment was atrial fibrillation. PCC was administered in 78% due to bleeding and in 22% of the cases to prepare patients for surgery. The mean administered doses were approximately 800IU. Pre-treatment INR determination was 4,4 in the warfarin group and 5,6 in the acenocoumarol group. In 26% of the patients it was not possible to have an absolute INR value (INR>10), 12 patients in the warfarin group (26% of the group) and 1 in the acenocoumarol group (14% of the group). After treatment all patients had a measurable INR and 37% of them had an INR ≤1,5. The INR values dropped to 1,9 in the acenocoumarol group and to 2 in the warfarin group. 18 patients received concomitantly i.v. vitamin K administration. 17 of them 10mg and one 20mg. We compared patients who only received PCC (33 patients) with those that received PCC and vitamin K (14 patients) and the impact on mortality. We excluded patients who did concomitant treatment with plasma (7 patients). We found a mortality of 36% in both groups, but we must point out their non-uniformity. None of the patients enrolled presented reported thrombotic complications. Patients presented different bleeding sites: 23% intracranial (67% mortality), 40% gastrointestinal (38% mortality) and 37% other (33% mortality). The overall mortality was 37%. Bleeding patients presented a mortality of 43% compared with no bleeding that presented mortality of 21%. Summary/Conclusions This study shows that Octaplex® treatment is efficient reversing the INR in patients taking oral VKA. In this population we didn’t find any difference, regarding overall mortality, between patients who had taken vitamin K plus PCC and those who had only taken PCC. We found a higher mortality rate in these bleeding patients.
- Transfusão Permuta Parcial em Crianças e Jovens com Doença Falciforme: Comparação da Experiência Manual com o Procedimento AutomatizadoPublication . Escobar, C; Moniz, M; Nunes, P; Abadesso, C; Ferreira, T; Barra, A; Lichtner, A; Loureiro, H; Dias, A; Almeida, HINTRODUCTION: The benefits of manual versus automated red blood cell exchange have rarely been documented and studies in young sickle cell disease patients are scarce. We aim to describe and compare our experience in these two procedures. MATERIAL AND METHODS: Young patients (≤ 21 years old) who underwent manual- or automated-red blood cell exchange for prevention or treatment of sickle cell disease complications were included. Clinical, technical and hematological data were prospectively recorded and analyzed. RESULTS: Ninety-four red blood cell exchange sessions were performed over a period of 68 months, including 57 manual and 37 automated, 63 for chronic complications prevention, 30 for acute complications and one in the pre-operative setting. Mean decrease in sickle hemoglobin levels was higher in automated-red blood cell exchange (p < 0.001) and permitted a higher sickle hemoglobin level decrease per volume removed (p < 0.001), while hemoglobin and hematocrit remained stable. Ferritin levels on chronic patients decreased 54%. Most frequent concern was catheter outflow obstruction on manual-red blood cell exchange and access alarm on automated-red blood cell exchange. No major complication or alloimunization was recorded. DISCUSSION: Automated-red blood cell exchange decreased sickle hemoglobin levels more efficiently than manual procedure in the setting of acute and chronic complications of sickle cell disease, with minor technical concerns mainly due to vascular access. The threshold of sickle hemoglobin should be individualized for clinical and hematological goals. In our cohort of young patients, the need for an acceptable venous access was a limiting factor, but iron-overload was avoided. CONCLUSION: Automated red blood cell exchange is safe and well tolerated. It permits a higher sickle hemoglobin removal efficacy, better volume status control and iron-overload avoidance.
- Utilização de Concentrado de Complexo Protrombínico na reversão de hipocoagulação oralPublication . Silva, S; Almeida, M; Oliveira, V; Gouveia, S; Lichtner, A; Mendes, DIntrodução: Os doentes sob hipocoagulação oral podem necessitar de reversão em caso de hemorragia ativa ou procedimentos emergentes. Perante hemorragias graves, os doentes sob anticoagulantes orais diretos (DOAC’s) parecem cursar mais favoravelmente do que doentes sob antivitamínicos K. Existe maior incidência de hemorragia digestiva no primeiro grupo, no entanto esta não parece alterar a mortalidade. Na ausência de antídotos específicos, é recomendada a utilização de concentrado de complexo protrombínico (CCP). Objetivos: Avaliar a utilização de CCP para reversão de hipocoagulação oral durante o ano de 2017, no nosso hospital. Métodos: Consulta dos registos do nosso serviço relativamente à utilização de CCP em 2017. Pesquisa dos processos clínicos dos doentes e recolha dos dados clínico-laboratoriais relevantes. Resultados: Foram avaliados 59 doentes, 62,71% sob varfarina e 37,29% sob DOAC’s. Em ambos os grupos, a maioria era do género masculino e com idade superior a 70 anos (65% vs. 55% e 62% vs. 77%, respetivamente). O principal motivo para anticoagulação era fibrilhação auricular não valvular (68% vs. 88%). Todos os doentes apresentavam hemorragia ativa grave. Mais de metade apresentava função renal alterada (57% vs. 59%). Cerca de 1/3 necessitaram de outros componentes sanguíneos /hemoderivados (32,4% vs. 36%). A mortalidade foi semelhante nos dois grupos (27% vs. 23%). Conclusão: Na nossa experiência a reversão de anticoagulação com complexo protrombínico (CCP) demonstrou resultados equivalentes, tanto em doentes sob varfarina como DOAC’s. A mortalidade e o consumo de componentes sanguíneos/hemoderivados foram semelhantes em ambos os grupos. Contudo, a elevada prevalência de disfunção renal nesta amostra poderá enviesar os resultados. A prescrição adequada e acompanhamento dos doentes hipocoagulados é fundamental.